BACKGROUND: Although congenital portosystemic shunts (CPSSs) are increasingly being recognized, the optimal treatment strategies and natural prognosis remain unclear, as individual CPSSs show different phenotypes.
METHODS: The medical records of 122 patients who were diagnosed with CPSSs at 15 participating hospitals in Japan between 2000 and 2019 were collected for a retrospective analysis based on the state of portal vein (PV) visualization on imaging.
RESULTS: Among the 122 patients, 75 (61.5%) showed PV on imaging. The median age at the diagnosis was 5 months. The main complications related to CPSS were hyperammonemia (85.2%), liver masses (25.4%), hepatopulmonary shunts (13.9%), and pulmonary hypertension (11.5%). The prevalence of complications was significantly higher in patients without PV visualization than in those with PV visualization (P < 0.001). Overall, 91 patients (74.6%) received treatment, including shunt closure by surgery or interventional radiology (n = 82) and liver transplantation (LT) or liver resection (n = 9). Over the past 20 years, there has been a decrease in the number of patients undergoing LT. Although most patients showed improvement or reduced progression of symptoms, liver masses and pulmonary hypertension were less likely to improve after shunt closure. Complications related to shunt closure were more likely to occur in patients without PV visualization (P = 0.001). In 25 patients (20.5%) without treatment, those without PV visualization were significantly more likely to develop complications related to CPSS than those with PV visualization (P = 0.011).
CONCLUSIONS: Patients without PV visualization develop CPSS-related complications and, early treatment using prophylactic approaches should be considered, even if they are asymptomatic.
METHODS: Level III.

Neurological symptoms that occur after treatment of portosystemic shunts, in cats, known as post-attenuation neurological signs (PANS) can be quite severe. This study seeks to analyze a better understanding of the neurological outcomes that result from reducing portosystemic shunts in felines and provide insights that could guide future clinical approaches and treatment strategies for congenital portosystemic shunts (CPSS).The research utilized the MOOSE Checklist as a guide. PubMed/MEDLINE, Web of Science ScienceDirect, Embase, Scopus, ProQuest, and Google Scholar were used. The study investigated diversity using variance, Cochran Q tests with Applied fixed effects, and random effects models. A meta-regression model identified contributors. Eggers test funnel plot and Beggs test for asymmetry addressed publication bias. 12 high-quality studies were discovered from 664 research papers. This research covered years, shunt morphology, and surgery. PANS occurred 38.9 % of the time in cats, while PAS occurred 20.2 %. The overall PANS mortality rate was 17 %, while PAS was 37.2 %. The complete ligation technique was most common in subgroup analysis. PANS occurrence ranged from 26.8 % to 56.5 % in cats with congenital extrahepatic portosystemic shunts The cause of PANS in cats is still unknown, and there is only limited evidence to justify the use of preventive antiepileptic medications such as levetiracetam. The treatment primarily aims to control neurologic symptoms, and the long-term outlook varies, with the potential for the reappearance of symptoms.

The prevalence of anatomical-based subtypes of feline congenital extrahepatic portosystemic shunts (EHPSS) has not been completely elucidated. The goal of this study was to use CT angiography to create an anatomical-based nomenclature system for feline congenital EHPSS. Additionally, subjective portal perfusion scores were generated to determine if intrinsic portal vein development was associated with different shunt conformations or patient age at the time of CT. The SVSTS and VIRIES list services were used to recruit cases. Data collected included patient DOB, gender, breed, weight, CT date, and reported diagnosis. Shunts were classified based upon (1) the shunt portal vessel(s) of origin, (2) the shunt systemic vessel(s) of insertion, and (3) any substantial portal vessels contributing to the shunt. Additionally, hepatic portal perfusion was subjectively scored between 1 (poor/none) and 5 (good/normal) based on the caliber of the intrahepatic PVs. A total of 264 CT scans were submitted from 29 institutions. Due to exclusion criteria, 33 (13%) were removed, leaving 231 CT scans to be included. Twenty-five different EHPSS anatomies were identified with five classifications accounting for 78% of all shunts (LGP [53%], LGC-post [11%], LCG [7%], LGC-pre [4%], and PC [4%]). Shunt origin involved the left gastric vein in 75% of the described classifications. Significant differences were identified among the five most common shunt types with respect to age at the time of CT scan (P = .002), breed (P < .001), and subjective portal perfusion score (P < .001). This refined anatomical classification system for feline EHPSS may enable improved understanding, treatment comparisons, and outcome prediction for cats with these anomalies.

The main objective was to conduct a prospective study reporting the outcome for dogs with an extrahepatic congenital portosystemic shunt (CPSS) treated with a \'complete ligation where possible\' philosophy. The second aim was to compare the outcomes following complete (C) polypropylene suture ligation versus partial thin film band (TFB) attenuation of a CPSS in dogs. Dogs that could not tolerate acute complete shunt ligation at surgery received partial shunt attenuation with TFB. Peri-operative complications, mortality, follow-up imaging findings, pre- and post-operative bile acid stimulation test results and details of any revision surgery performed were recorded. A follow-up health-related quality of life questionnaire enabled the calculation of a postoperative clinical shunt score, a quality of life score, and determined if any dogs were still on a hepatic diet and/or other medical management at a minimum of 6 months after surgery. Of the 110 dogs enrolled, 57 received complete ligation and 53 received partial TFB attenuation. Peri-operative mortality, the occurrence of post-attenuation neurological complications, the occurrence of multiple acquired shunts, the postoperative clinical shunt score and quality of life score were not significantly different between the two groups. Dogs in the C group were older, heavier and demonstrated a greater number of shunt classifications where the entry into the systemic circulation was the phrenic vein or azygous vein. Dogs in the TFB group had a greater number of unchanged bile acid concentrations after surgery, were more likely to remain on the hepatic diet and/or medical management after surgery and underwent a greater number of revision surgeries. There was variability in the precision of both ultrasound and computed tomographic angiography follow-up imaging compared to intra-operative mesenteric portovenography findings at revision surgery. Overall, dogs with an extrahepatic portosystemic shunt receiving either complete acute shunt ligation or partial TFB shunt attenuation are expected to have an excellent long-term clinical outcome and there is no reason to suggest that a dog able to tolerate complete acute shunt closure should be denied the benefit of this.

To evaluate recovery of platelet count after transjugular intrahepatic portosystemic shunt (TIPS) creation and patient factors predicting platelet recovery after TIPS creation.
Adults with cirrhosis who underwent TIPS creation at 9 U.S. hospitals from 2010 to 2015 were included in this retrospective analysis. Change in platelets from before TIPS to 4 months after TIPS creation was characterized. Logistic regression was used to assess factors associated with top quartile percentage platelet increase after TIPS. Subgroup analyses were performed among patients with a pre-TIPS platelet count of ≤50 ×109/L.
A total of 601 patients were included. The median absolute change in platelets was 1 × 109/L (-26 × 109/L to 25 × 109/L). Patients with top quartile percent platelet increase experienced ≥32% platelet increase. In multivariable analysis, pre-TIPS platelet counts (odds ratio [OR], 0.97 per 109/L; 95% CI, 0.97-0.98), age (OR, 1.24 per 5 years; 95% CI, 1.10-1.39), and pre-TIPS model for end-stage liver disease (MELD) scores (OR, 1.06 per point; 95% CI, 1.02-1.09) were associated with top quartile (≥32%) platelet increase. Ninety-four (16%) patients had a platelet count of ≤50 × 109/L before TIPS. The median absolute platelet change was 14 × 109/L (2 × 109/L to 34 × 109/L). Fifty-four percent of patients in this subgroup were in the top quartile for platelet increase. In multivariable logistic regression, age (OR, 1.50 per 5 years; 95% CI, 1.11-2.02) was the only factor associated with top quartile platelet increase in this subgroup.
TIPS creation did not result in significant platelet increase, except among patients with a platelet count of ≤50 × 109/L before TIPS. Lower pre-TIPS platelet counts, older age, and higher pre-TIPS MELD scores were associated with top quartile (≥32%) platelet increase in the entire cohort, whereas only older age was associated with this outcome in the patient subset with a pre-TIPS platelet count of ≤50 × 109/L.

OBJECTIVE: The purpose of this study was to evaluate whether concomitant left gastric vein embolization (LGVE) during transjugular intrahepatic portosystemic shunt (TIPS) for acute variceal hemorrhage could reduce the risk of bleeding recurrence.
METHODS: A national multicenter observational study was conducted in 14 centers between January 2019 and December 2020. All cirrhotic patients who underwent TIPS placement for acute variceal bleeding were included. During TIPS procedure, size of left gastric vein (LGV), performance of LGVE, material used for LGVE and portosystemic pressure gradient (PPG) before and after TIPS placement were collected. A propensity score for the occurrence of LGVE was calculated to assess effect of LGVE on rebleeding recurrence at six weeks and one year.
RESULTS: A total of 356 patients were included (mean age 57.3 ± 10.8 [standard deviation] years; 283/356 [79%] men). Median follow-up was 11.2 months [interquartile range: 1.2, 13.3]. The main indication for TIPS was pre-emptive TIPS (162/356; 46%), rebleeding despite secondary prophylaxis (105/356; 29%), and salvage TIPS (89/356; 25%). Overall, 128/356 (36%) patients underwent LGVE during TIPS procedure. At six weeks and one year, rebleeding-free survival did not differ significantly between patients who underwent LGVE and those who did not (6/128 [5%] vs. 15/228 [7%] at six weeks, and 11/128 [5%] vs. 22/228 [7%] at one year, P = 0.622 and P = 0.889 respectively). A total of 55 pairs of patients were retained after propensity score matching. In patients without LGVE, the rebleeding rate was not different from those with LGVE (3/55 [5%] vs. 4/55 [7%], P > 0.99, and 5/55 [9%] vs. 6/55[11%], P > 0.99, at six weeks and one year respectively). Multivariable analysis identified PPG after TIPS placement as the only predictor of bleeding recurrence (hazard ratio = 1.09; 95% confidence interval: 1.02-1.18; P = 0.012).
CONCLUSIONS: In this multicenter national real-life study, we did not observe any benefit of concomitant LGVE during TIPS placement for acute variceal bleeding on bleeding recurrence rate.

UNASSIGNED: To design a prospective study on endovascular closure of congenital portosystemic shunts. The primary endpoint was to assess the safety of endovascular closure. The secondary endpoint was to evaluate the clinical, analytical and imaging outcomes of treatment.
UNASSIGNED: Fifteen patients (age range: 2 days to 21 years; 10 male) were referred to our center due to congenital portosystemic shunts. The following data were collected prior to treatment: age, sex, medical history, clinical and analytical data, urine trimethylaminuria, abdominal-US, and body-CT. The following data were collected at the time of intervention: anatomical and hemodynamic characteristics of the shunts, device used, and closure success. The following data were collected at various post-intervention time points: during hospital stay (to confirm shunt closure and detect complications) and at one year after (for clinical, analytical, and imaging purposes).
UNASSIGNED: The treatment was successful in 12 participants, migration of the device was observed in two, while acute splanchnic thrombosis was observed in one. Off-label devices were used in attempting to close the side-to-side shunts, and success was achieved using Amplatzer™ Ductus-Occluder and Amplatzer™ Muscular-Vascular-Septal-Defect-Occluder. The main changes were: increased prothrombin activity (p=0.043); decreased AST, ALT, GGT, and bilirubin (p=0.007, p=0.056, p=0.036, p=0.013); thrombocytopenia resolution (p=0.131); expansion of portal veins (p=0.005); normalization of Doppler portal flow (100%); regression of liver nodules (p=0.001); ammonia normalization (p=0.003); and disappearance of trimethylaminuria (p=0.285).
UNASSIGNED: Endovascular closure is effective. Our results support the indication of endovascular closure for side-to-side shunts and for cases of congenital absence of portal vein.

Congenital portosystemic shunt (CPSS) is a rare disorder characterized by a diversion of Porto-mesenteric blood into systemic veins. Type I is an end to side fistula between the portal vein and the inferior vena cava. Type II is a side to side fistula between the main portal vein or its branches and mesenteric, splenic, gastric, and systemic veins. Clinical presentation of these patients varies. Treatment and management are guided by the type of malformation and clinical presentation. Herein, we present a case of CPSS Type IIb with neurological symptoms, treated with endovascular occlusion with 6-year follow-up without remission.

Hepatic myelopathy is a complication seen in patients with chronic liver failure with physiologic or iatrogenic portosystemic shunting. The main symptom is progressive lower limb dyskinesia. The role of the brain motor control center in hepatic myelopathy is unknown. This study aimed to investigate the gray matter changes in patients with hepatic myelopathy secondary to transjugular intrahepatic portosystemic shunt and to examine their clinical relevance. This was a cross-sectional study. Twenty-three liver failure patients with hepatic myelopathy (hepatic myelopathy group), 23 liver failure patients without hepatic myelopathy (non-hepatic myelopathy group) after transjugular intrahepatic portosystemic shunt, and 23 demographically matched healthy volunteers were enrolled from March 2014 to November 2016 at Xijing Hospital, Air Force Military Medical University (Fourth Military Medical University), China. High-resolution magnetization-prepared rapid gradient-echo brain imaging was acquired. Group differences in regional gray matter were assessed using voxel-based morphometry analysis. The relationship between aberrant gray matter and motor characteristics was investigated. Results demonstrated that compared with the non-hepatic myelopathy group, gray matter volume abnormalities were asymmetric, with decreased volume in the left insula (P = 0.003), left thalamus (P = 0.029), left superior frontal gyrus (P = 0.006), and right middle cingulate cortex (P = 0.021), and increased volume in the right caudate nucleus (P = 0.017), corrected with open-source software. The volume of the right caudate nucleus in the hepatic myelopathy group negatively correlated with the lower limb clinical rating of the Fugl-Meyer Assessment (r = -0.53, P = 0.01). Compared with healthy controls, patients with and without hepatic myelopathy exhibited overall increased gray matter volume in both thalami, and decreased gray matter volume in both putamen, as well as in the globus pallidus, cerebellum, and vermis. The gray matter abnormalities we found predominantly involved motor-related regions, and may be associated with motor dysfunction. An enlarged right caudate nucleus might help to predict weak lower limb motor performance in patients with preclinical hepatic myelopathy after transjugular intrahepatic portosystemic shunt. This study was approved by the Ethics Committee of Xijing Hospital, Air Force Military Medical University (Fourth Military Medical University), China (approval No. 20140227-6) on February 27, 2014.